Preoperative platelet-to-lymphocyte ratio is a predictor of prognosis in patients with ampullary carcinoma.

AIM: To emphasize the significance of the platelet-to-lymphocyte ratio (PLR) in estimating the postoperative prognosis or survival measures in patients with carcinoma of the ampulla of Vater. METHODS: We retrospectively reviewed 82 patients, who underwent pancreaticoduodenectomy for ampullary carcinoma between July 2001 and April 2014. We investigated the predictive significance of the preoperative PLR for disease-free survival (DFS) or overall survival (OS). The possible correlations between the PLR and clinical or pathological features were also evaluated. RESULTS: The 5-year DFS and OS rates of the patients with carcinoma of the ampulla of Vater after pancreaticoduodenectomy were 51 % and 64 %, respectively. Multivariate analysis revealed a significantly worse OS in patients with a PLR ≥ 212 [hazard ratio (HR): 3.446; 95% confidence interval (CI): 1.4-8.43; p = 0.007], lymphovascular invasion (HR: 2.973; 95% CI: 1.25-7.03; p = 0.013), or pathological stage pT3/4 (HR: 2.761; 95% CI: 1-7.1; p = 0.035). Similarly, DFS was significantly worse in patients with lymphovascular invasion (HR: 2.24; 95% CI: 1.1-4.56; p = 0.025) or stage pT3/4 (HR: 2.243; 95% CI, 1.03-4.84; p = 0.04). CONCLUSION: The preoperative PLR shows a predictive significance for the prognosis of postoperative patients with carcinoma of the ampulla of Vater. We suggest that because of its predictive value, the PLR can be used in the development of further approaches to monitor and manage patients with poor prognosis Tab. 4, Fig. 1, Ref. 45).

Brentuximab vedotin for relapsed or refractory Hodgkin lymphoma: experience in Turkey.

Current treatment modalities can cure up to 70-80 % of patients with classical Hodgkin lymphoma. Approximately, 20-30 % of patients require further treatment options. Brentuximab vedotin has been approved for the treatment of relapsed and refractory Hodgkin lymphoma. In the present study, we report the experience with brentuximab vedotin as single agent in 58 patients with relapsed or refractory Hodgkin lymphoma. The objective response rate was 63.5 % with 13 complete responders (26.5 %) among 49 patients evaluated at the early phase of treatment (2-5 cycles). Upon treatment prolongation (≥6 cycles), 37 patients achieved a final objective response rate of 32.4 % with 21.6 % of complete and 10.8 % of partial response. Overall survival at 12 months was 70.6 %, and progression-free survival at 12 months was 32.8 %. Median overall survival could not be reached and median progression-free survival was 7 months. While the median duration of response was 9 months in the whole cohort, it was 11.5 months in the complete responders. Complete response rates in patients treated with >3 chemotherapy regimens before brentuximab vedotin were significantly lower (p = 0.016). Fourteen patients were subsequently transplanted. In conclusion, brentuximab vedotin provided a bridge to transplantation in approximately one quarter of the patients. The declining response rates during the course of treatment suggest that transplantation should be implemented early during brentuximab vedotin treatment.

A Very Rare Case -  Hairy Cell Leukemia in Patient with Sarcoidosis.

Although the coexistence of hairy cell leukemia with sarcoidosis has been reported in a few cases in the literature, in our case the patient had been diagnosed and followed about 10 years with sarcoidosis and massive splenomegaly. It has been demonstrated that T helper 1 cells exist in organs influenced by sarcoidosis. These cells produce IL-2 and IFN-γ and induce a nonspecific inflammatory response and granuloma formation. Also these cytokines may play a role in the development of hairy cell leukemia.Key words: hairy cell leukemia -  sarcoidosis - massive splenomegaly.

Increased expression of exon 2 deleted MEFV transcript in familial Mediterranean fever patients.

A defect in MEFV gene expression regulation has been implicated in familial Mediterranean fever (FMF) etiopathophysiology. Here we show significantly higher expression level in second exon lacking MEFV transcript in FMF patients compared with healthy controls (P=0.026). Our results also point out a possible role of exon 2 deleted MEFV transcript in FMF pathogenesis.

Low plasma apelin levels in newly diagnosed type 2 diabetes mellitus.

OBJECTIVE: To investigate blood apelin concentrations in patients with newly diagnosed and untreated type 2 diabetes mellitus (T2DM) who had no additional disorder and to investigate the association of apelin with adiponectin, body mass indexes (BMI) and insulin sensitivity. METHODS: Forty patients with T2DM and 40 healthy controls were enrolled. Apelin levels were measured along with BMI, lipids, glucose, insulin and adiponectin levels, and HOMA-IR indexes. Age, sex and BMI were similar in the two groups. RESULTS: Plasma apelin and adiponectin levels were significantly lower in the diabetic group compared to controls (p<0.001, for both). Insulin levels and HOMA indexes were significantly higher in patients with T2DM (p<0.001 and p=0.001, respectively). Apelin levels were negatively correlated with age (r=-0.315, p=0.006), fasting blood glucose (r=-0.556, p<0.001) and HOMA indexes (r=-0.411, p=0.001), and positively correlated with plasma adiponectin levels (r=0.593, p<0.001). Plasma adiponectin was negatively correlated to plasma insulin (r=-0.379, p=0.001), fasting glucose (r=-0.604, p<0.001), HOMA-IR (r=-0.559, p<0.001) and BMI (=-0.229, p=0.04). CONCLUSIONS: Plasma apelin is reduced in newly diagnosed and untreated patients with T2DM having no confounders. Regulation of circulating apelin and adiponectin seems to be in the same manner in patients with T2DM. Dysregulation of apelin might be involved in the mechanism of establishment of overt diabetes mellitus as well as associated atherosclerotic complications.

Plasma apelin is lower in patients with elevated LDL-cholesterol.

Hypercholesterolemia is a major risk factor for atherosclerosis. Dysregulation of adipokines contribute to atherosclerotic diseases. Apelin has recently been shown to be secreted by the adipose tissue in association with hyperinsulinemia and inflammation. We searched plasma apelin levels in patients with elevated low density lipoprotein (LDL)-cholesterol having no additional disorder. Thirty-three patients with hypercholesterolemia and 50 age-, sex-, and body mass index-matched healthy controls were evaluated for their apelin, adiponectin and high sensitivity C-reactive protein (hsCRP) levels, and homeostasis model assessment (HOMA) indexes. Plasma apelin-12 and adiponectin were determined by ELISA and RIA, respectively. Plasma apelin levels were lower in patients with elevated LDL-cholesterol compared to healthy controls (p<0.001). Plasma adiponectin concentration was also lower in the dyslipidemic patients (p<0.001). hsCRP levels were similar in the two groups. Fasting plasma glucose was normal in both groups. HOMA indexes in the dyslipidemic group were higher than the controls (p=0.005). A mild to moderate negative correlation with HOMA and positive correlation with high density lipoprotein cholesterol of apelin was found in the dyslipidemic group. Plasma apelin is decreased in non-obese, non-diabetic and normotensive patients with elevated LDL-cholesterol. Low apelin levels in hypercholesterolemia seem associated with insulin resistance, which needs to be investigated in larger populations as well as in other atherosclerotic conditions.

Brucellar breast abscess.

Brucellosis is an endemic disease seen in many countries. It may affect different organ systems. Brucellar breast abscess is a rare entity. We report the radiological findings of breast abscess due to brucella. A 63-year-old female was investigated with mammography, ultrasonography, magnetic resonance imaging (MRI), and magnetic resonance spectroscopy (MRS). A mass measuring 25 x 20 x 15 mm was detected in the left breast on mammography and ultrasonography. The mass was homogenously hyperintense on T1- and T2-weighted MRI images. On contrast-enhanced T1-weighted images, peripheral capsular enhancement was found. MR spectroscopic analysis of the mass revealed elevated lipid and acetate peaks. The diagnosis was provided by fine needle aspiration biopsy and specimen culture. The lesion had diminished in size after 12 months' treatment with combined tetracycline and rifampicine.

Intestinal lymphangiectasia in a patient with Zellweger cerebrohepatorenal syndrome.

Zellweger cerebrohepatorenal syndrome (ZWCHRS) is an autosomal-recessive disease, characterized by the absence or profound deficiency of peroxisomes. We report a case of ZWCHRS with intestinal lymphangiectasia, observed as an autopsy finding. This combination is previously unreported.

Serum malondialdehyde concentration in babies with hyperbilirubinaemia.

AIM: To determine lipid peroxide concentrations in the first 10 days of life. METHODS: Malondialdehyde concentrations were investigated in neonates with or without hyperbilirubinaemia during the first 10 days of life. RESULTS: Serum malondialdehyde concentrations were higher in infants with hyperbilirubinaemia than in controls. A positive correlation was found between malondialdehyde and bilirubin concentrations in the study group. When the study group was categorised according to the presence of haemolysis, a significant correlation was found between malondialdehyde and bilirubin concentrations in those infants with hyperbilirubinaemia due to haemolysis. There was no such correlation in those without haemolysis. CONCLUSION: Exchange transfusion rapidly produces variable changes in pro-oxidant and antioxidant plasma concentrations in neonates, which may be responsible for free radical metabolism. The fall in malondialdehyde concentration is probably directly related to its exogenous removal by exchange transfusion.

Clinical and magnetic resonance imaging features of L-2-hydroxyglutaric acidemia: report of three cases in comparison with Canavan disease.

We report three cases of L-2-hydroxyglutaric acidemia and three cases of Canavan disease. The L-2-hydroxyglutaric acidemia cases are the first biochemically proven Turkish cases. Magnetic resonance imaging findings in the cases and similarities between the two diseases are emphasized. Both diseases are characterized by predominant subcortical white-matter involvement and dentate nuclei lesions with variable basal ganglia involvement. Canavan disease differs from L-2-hydroxyglutaric acidemia by the presence of typical brainstem involvement.

Lymphoma with bilateral cavernous sinus involvement in early childhood.

A 4-year-old girl developed complete ophthalmoplegia with intact pupillary responses. Computed tomography and magnetic resonance imaging demonstrated a mass invading the cavernous and sphenoid sinuses and posterior ethmoidal cells. Biopsy revealed non-Hodgkin lymphoma. This patient is the youngest reported with malignant lymphoma of the cavernous sinus and the second reported with bilateral cavernous sinus involvement.

Dermoid tumor with persistently low CSF glucose and unusual CT and MRI findings.

A patient with dermoid tumor, presenting with persistent hypoglycorrhachia in the absence of cerebrospinal fluid pleocytosis is reported. The presence of ring enhancements without infection, surrounding edema, and mass effect on computed tomography and magnetic resonance imaging are unusual findings for these tumors.

Investigation of ascorbate-Cu (II) induced cleavage of DNA by scanning tunneling microscopy.

Scanning Tunneling Microscopy (STM) was used for the investigation of oxidative DNA damage. A PCR amplified fragment of human beta-globin gene was used as a model for time dependent cleavage reaction by ascorbate and copper. Cleavage reactions were carried out in a medium containing 0.5 microgram/20 microliters DNA, 20 nM Tris-HC1 pH, 7.8 and ascorbate-Cu (II) in the final concentrations of 1 mM and 30 microM, respectively. The mixtures were incubated at 37 degrees C for 5, 15 and 30 min. For STM studies, 3 pg/5 microliters DNA samples were deposited on the gold coated mica and dried in a water flow vacuum drier. The STM was operated in air at atmospheric pressure with a tip-to-substrate bias of 100 mV and tunneling currents of < 10 pA. Etched tips of Pt/Ir wires were used in a constant current mode. The degradated DNA structure can be distinguished from the intact DNA and the sizes of the degradation products can be identified in the STM micrographs. The size of fragments decreased from approximately 3000 A to 34 A in ascorbate-Cu (II) medium, after 30 min of incubation.

Relation between the birthweight and cord, maternal serum and amniotic fluid growth hormone levels.

Maternal and cord serum and amniotic fluid growth hormone (GH) levels were measured in 55 term pregnancies in order to evaluate the role of GH in prenatal fetal somatic growth. The study group consisted of patients who had small, large, and average for gestational age infants. The difference in mean maternal and cord serum and amniotic fluid GH levels between the three weight groups were found to be insignificant. It is concluded that GH does not influence fetal somatic growth.

The use of IgM-enriched intravenous immunoglobulin for the treatment of neonatal sepsis in preterm infants.

The value of IgM-enriched immunoglobulin therapy in 44 preterm infants with neonatal sepsis was evaluated in a prospective randomized study. All infants received antibiotic therapy and fresh plasma and/or whole blood transfusions. Twenty randomly-chosen infants were allocated to receive 5 ml/kg/d of IgM-enriched immunoglobulin intravenously for three days. Although the mortality rate in preterm infants whose gestational ages were 31-34 weeks in the immunotherapy group was slightly lower than in the control group, the general mortality rate from sepsis in the control group (9/24) and in the immunotherapy group (6/20) showed no statistically significant difference (37.5% vs 30.0%, p < 0.05).

Plasma chromium levels in small-for-gestational-age newborn infants.

Plasma chromium (Cr) levels were determined in 24 preterms and 18 full-term newborn infants. There was no statistically significant differences in plasma Cr levels between the preterm and full-term infants. Plasma Cr levels were similar in small-for-gestational-age infants and in infants with hypoglycemia compared with healthy infants.

Breast milk beta-glucuronidase levels in hyperbilirubinemia.

Breast milk beta-glucuronidase was thought to be one of the etiological factors in the pathogenesis of late-onset breast-milk jaundice, but results of these studies are conflicting. In this study breast milk beta-glucuronidase levels were determined in groups with physiologic jaundice, early breast-feeding jaundice and late breast-milk jaundice. No difference in beta-glucuronidase levels of these three groups was found in samples taken on the 4th and 15th days of life. beta-glucuronidase activity in breast milk declined from the 4th to 15th day in all groups. These results imply that factors other than breast milk beta-glucuronidase activity should be investigated to reveal the pathogenesis of late-onset breast-milk jaundice.

Hepatic mucormycosis in a child with fulminant hepatic failure.

Mucormycosis is an uncommon, opportunistic infection in children. We present a case of fulminant hepatic failure with hepatic mucormycosis. Although the suggested defects and pathogenic mechanisms in infections related to hepatic failure and mucormycosis are similar, few cases with both mucormycosis and liver failure have been reported.

Multiple intracranial tuberculomas in a child.

Intracranial tuberculomas continue to be a serious complication of central nervous system tuberculosis. Tuberculomas are conglomerates of tubercles resulting from hematogenous spread of infection. Modern neuroimaging studies such as magnetic resonance imaging and molecular biologic techniques such as polymerase chain reaction are helpful in the diagnosis of central nervous system tuberculosis and tuberculomas. We report a boy with multiple intracranial tuberculomas diagnosed and treated with the aids of magnetic resonance and polymerase chain reaction techniques.

Serratia marcescens: an emerging microorganism in the neonatal intensive care unit.

As smaller babies survive in neonatal intensive care units, late-onset septicemia with unusual pathogens appears. Between 1 January and 31 December 1998, in Hacettepe University Ihsan Dogramaci Children's Hospital Neonatal Intensive Care Unit, seven infants had S. marcescens isolates. Four babies had septicemia with the microorganism. The case fatality rate was 50 percent in infants with S. marcescens septicemia. The combination of ceftazidime or imipenem with amikacin appears appropriate for the treatment of newborns with Serratia infection.